SSc

Question 1

Core pathogenesis of systemic sclerosis

Answer 1

Autoimmune microangiopathy + fibroblast overdrive → collagen overload → fibrosis (skin → lung → GI → heart → kidney)

Question 2

Main cytokine mediators in SSc

Answer 2

↑ TGF-β and Endothelin-1 → fibroblast activation → collagen deposition

Question 3

Typical demographic for SSc

Answer 3

Middle-aged women (30–60 y); F:M ≈ 4–6:1

Question 4

Leading cause of death in SSc

Answer 4

Interstitial lung disease (ILD) > PAH > renal crisis

Question 5

Key environmental risk factor for SSc

Answer 5

Silica exposure (strongest environmental link)

Question 6

Autoantibody for diffuse SSc

Answer 6

Anti-Scl-70 (topoisomerase I) → ILD / renal / cardiac involvement

Question 7

Autoantibody for limited SSc

Answer 7

Anti-centromere → PAH + CREST features

Question 8

Autoantibody predicting renal crisis

Answer 8

Anti-RNA polymerase III

Question 9

CREST acronym meaning

Answer 9

Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangiectasia

Question 10

Most common lung pattern in SSc

Answer 10

NSIP > UIP (ILD = main cause of death)

Question 11

Definition of scleroderma renal crisis

Answer 11

Malignant hypertension + acute kidney injury

Question 12

PFT pattern in SSc-ILD

Answer 12

Restrictive (↓ FVC, ↓ TLC, normal/increased FEV₁/FVC) + low DLCO

Question 13

PFT clue distinguishing PAH vs ILD

Answer 13

Low DLCO + normal volumes = PAH; Low DLCO + low FVC = ILD

Question 14

Thresholds predicting ILD progression

Answer 14

FVC < 70 % or > 20 % fibrosis on HRCT

Question 15

Annual FVC drop defining progression

Answer 15

> 10 % fall per year

Question 16

When to screen for PAH using DETECT

Answer 16

SSc > 3 years + DLCO < 60 %; screen annually

Question 17

Raynaud/digital ulcer therapy sequence

Answer 17

Nifedipine → Sildenafil → Iloprost → Bosentan

Question 18

First-line treatment for SSc-ILD

Answer 18

Mycophenolate mofetil (MMF)

Question 19

Add-on anti-fibrotic for ILD

Answer 19

Nintedanib – slows FVC decline

Question 20

Biologic for early inflammatory dcSSc

Answer 20

Tocilizumab (anti-IL-6R) – preserves FVC

Question 21

Second-line biologic for refractory ILD

Answer 21

Rituximab (anti-CD20) – depletes B cells

Question 22

Combination therapy for SSc-PAH

Answer 22

Ambrisentan + Tadalafil (AMBITION strategy)

Question 23

Immediate management of renal crisis

Answer 23

Start ACEi (Captopril) even if Cr rises; avoid high-dose steroids

Question 24

GI/Nutrition management in SSc

Answer 24

PPI ± Prokinetic for reflux; Rifaximin for SIBO

Question 25

MSK/overlap management

Answer 25

Pred ≤10 mg + DMARD (MTX/MMF)

Question 26

Curative option for rapidly progressive dcSSc

Answer 26

Autologous HSCT (↑ survival; TRM 7–10 %)

Question 27

Key exam steroid trap

Answer 27

High-dose steroids precipitate renal crisis

Question 28

When not to use ACEi

Answer 28

Avoid prophylactic ACEi – increases risk of renal crisis

Question 29

Antibody–organ map mnemonic

Answer 29

RNA pol III → renal | Scl-70 → lung | Centromere → PAH

Question 30

PFT clue for PAH

Answer 30

Low DLCO with normal lung volumes

Question 31

Best predictor of extensive ILD

Answer 31

FVC < 70 % or CT fibrosis > 20 %

Question 32

Most effective PAH combo

Answer 32

Ambrisentan + Tadalafil

Question 33

Classic diagnostic feature for SSc

Answer 33

Skin thickening proximal to MCP joints (9 points = diagnostic)

Question 34

ANA-negative SSc significance

Answer 34

Severe vasculopathy variant (rare)

Question 35

Key poor prognostic markers

Answer 35

Male sex, dcSSc, RNA pol III, PAH, cardiac disease, rapid skin progression

Question 36

5-year survival rate

Answer 36

Approximately 80 %

Question 37

Exam mnemonic

Answer 37

“Vessel → Fibrosis → Failure” + “ACE in crisis, not before”